Practice of noninvasive ventilation for cystic fibrosis: a nationwide survey in France.
BACKGROUND
No guidelines are available for noninvasive ventilation (NIV) for cystic fibrosis (CF).


OBJECTIVE
To survey and evaluate the use of NIV for CF in France.


METHODS
We surveyed the coordinator physicians of every accredited CF center in France.


RESULTS
The respondents represented 36 centers (15 pediatric centers, 13 adult centers, and 8 centers that see both pediatric and adult patients), which had a total of 4,416 patients with CF at the time of the study, 168 (3.8%) of whom were using NIV. NIV was being used more often in the adults centers (7.6% of these patients) than in the pediatric centers (1.2% of these patients) or adult-and-pediatric centers (4.1% of these patients) (P= .01). All the respondent centers use NIV as first-line treatment for severe hypercapnic respiratory exacerbation and for stable diurnal hypercapnia, especially when associated with sleep disturbance. Bi-level pressure-targeted ventilation is the preferred ventilation mode. Settings are adjusted based on arterial blood gas values, noninvasive evaluation of patient-ventilator synchrony, patient comfort, and sometimes a sleep study. The surveyed centers reported a number of expected benefits from NIV, but few of those benefits have been proven. Problems with NIV are common and limit its use.


CONCLUSIONS
We found a relative homogeneity in these French centers' stated indications for and use of NIV, which highlights their numerous expectations about the benefits of NIV, which contrasts with the few validated benefits. Studies of the benefits of NIV are needed.
Fauroux
Brigitte
B
Pediatric Pulmonary Department, Hôpital Armand Trousseau, Centre de Ressources et de Compe'tences pour la Mucoviscidose, Université Pierre et Marie Curie-Paris 6, Paris, France. brigitte.fauroux@trs.aphp.fr
Burgel
Pierre-Régis
PR
Boelle
Pierre-Yves
PY
Cracowski
Claire
C
Murris-Espin
Marlène
M
Nove-Josserand
Raphaele
R
Stremler
Nathalie
N
Derlich
Laurence
L
Giovanetti
Philippe
P
Clément
Annick
A
D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000628:therapy*; D005500:Follow-Up Studies; D005602:France; D019538:Health Care Surveys; D006801:Humans; D017063:Outcome Assessment, Health Care; D017410:Practice Guidelines as Topic; D012121:Respiration, Artificial / Q000592:standards / Q000706:statistics & numerical data*; D012124:Respiratory Care Units / Q000592:standards / Q000706:statistics & numerical data*; D012189:Retrospective Studies